Medicina (Kaunas) 2006; 42(5)
401
KLINIKINIS ATVEJIS
Neurosyphilis manifesting as spinal transverse myelitis
Vaidas Matijošaitis, Antanas Vaitkus, Valius Pauza, Skaidra Valiukevičienė1, Rymantė Gleiznienė2
Clinic of Neurology, 1Clinic of Skin and Sexually Transmitted Diseases,
2
Clinic of Radiology, Kaunas University of Medicine, Lithuania
Key words: myelitis, neurosyphilis, magnetic resonance imaging.
Summary. Spinal myelitis caused by neurosyphilis is an extremely rare disease, and there are
only few visual examples of magnetic resonance imaging scans. We present a clinical case of
neurosyphilis, which is of great importance concerning diagnostic, differential diagnosis, and
tactics of management. A patient complaining of progressive legs weakness, numbness, and
shooting-like pain in the legs as well as pelvic dysfunction was admitted to the hospital.
Neurological examination revealed spinal cord lesion symptoms: legs weakness, impairment of
superficial and deep sensation together with pathological symptoms in the legs. Hernia of
intervertebral disc or tumor was suspected, and myelography with computed tomography of the
spine was performed. No pathological findings were observed. More precise examination of the
patient (a small scar in the genitals and condylomata lata in anal region were noticed) pointed to
possible syphilis-induced spinal cord lesion. Serologic syphilis diagnostic tests (Treponema
pallidum hemagglutination assay, reagin plasma response, serum enzyme-linked immunosorbent
assay) and cerebrospinal fluid tests (general cerebrospinal fluid test and Venereal Disease
Research Laboratory test) confirmed the diagnosis of neurosyphilis. Spinal cord lesion determined
by magnetic resonance imaging was evaluated as spinal syphilis or syphilis-induced myelitis.
Conventional treatment showed a partial effect.
Introduction
Syphilis is a chronic infectious disease caused by
the bacterium Treponema pallidum. It is usually acquired by sexual contact with another infected individual. It progresses, if untreated, through primarily,
secondary, and tertiary stages. In about 30% of
untreated patients late disease of the heart, central
nervous system or other organs develops (1). Neurosyphilis can be asymptomatic or cause different disorders, like tabes dorsalis, general paresis, and meningovascular syphilis. There are also two types of spinal
syphilis other than tabes dorsalis: syphilitic meningomyelitis and meningovascular syphilis. Neurological
symptoms can appear in any stage of syphilis. During
secondary syphilis lesion of central nervous system
develops in up to 40% of the patients.
The incidence of syphilis decreased significantly
with the introduction of penicillin in the 1940s but
rose sharply again with the advent of human immunodeficiency virus (HIV) infection in the 1980s. Despite the overall decreases, outbreaks of syphilis have
recently been reported in men who have sex with men.
Some epidemiological studies suggest that the incidence of primary syphilis is rising (2, 3). The prevalence of syphilis in Lithuania decreased over the last
years from 101.4 cases per 100,000 in 1996 to 9.9
cases per 100,000 in 2004 (4).
This clinical case report is important not only
because of its clinical value, but also it has scientific
value. Despite significant scientific achievements in
biology, not all biological features of treponemes are
known. Nervous system lesions caused by syphilis are
not so frequent to draw inferences about them from
larger patient groups. This makes every case important
for the future conclusions.
Presentation of the case
A 38-year-old man was admitted to the hospital
because of shooting low back pain, radiating to the
legs, legs weakness, numbness, and retained urination
with constipation. The patient had been well until four
months ago, when he felt that his legs became weaker
Correspondence to A. Vaitkus, Clinic of Neurology, Kaunas University of Medicine, Eivenių 2, 50009 Kaunas,
Lithuania. E-mail: [email protected]
Vaidas Matijošaitis, Antanas Vaitkus, Valius Pauza et al.
402
and he could not walk as good as before. He thought
that he got cold while digging a well because of
working in cool and wet environment. Weakness and
numbness in his legs deteriorated, and one month
before admission to the hospital the patient could not
walk. Sharp shooting pain radiating to his legs appeared. Couple days before the admission patient
began having difficulty emptying his bladder and had
constipation. There was no history of a tick bite, head
injury, seizure disorder, recent viral illness, diabetes,
hypertension, and intravenous drug abuse. Patient
denied having accidental sexual relations. His girlfriend died one and a half years ago.
The patient did not have fever, chills, cough, rash,
weight loss, or neurological symptoms related to the
arms. Patient was jobless and got only some occasional
utility works. The temperature was 36.7oC, the pulse
was 72 beats/minute, and the respirations were 24
times per minute. His blood pressure was 120/80
mmHg. On general physical examination, the patient
appeared well.
Neurological examination showed normal motor
function in the arms. Muscle strength in the legs was
3/5 in the proximal and distal muscle groups bilaterally. The deep-tendon reflexes were + + + in the
arms; the knee jerks were brisk + + +; ankle jerks
were diminished, + bilaterally. Abdominal reflexes
were absent. Pinprick and thermal sensations were
absent below the costal margins. Joint-position sense
and vibratory sensation were absent in the legs.
Pathological Babinski signs were observed in both
feet. Straight-leg rising to an angle of 70 degrees
provoked hamstring pain on both sides. Coordination
was normal in the arms but poor in the legs. The patient
could barely walk unsupported. A small scar was
noticed on the dorsal surface of the penis. Later on,
condylomata lata in anal region were observed. Neurological symptoms (loss of pain and thermal sensations bellow costal margins, impaired deep sensations in the legs as well as muscle weakness in the
legs, pathological Babinski symptoms, and impaired
urination together with bowel function) pointed to
spinal cord lesion localized in the thoracic area. Intervertebral disc herniation of this area was suspected
with further differentiation of other possible causes
of spinal cord injury (spinal tumor, abscess, multiple
sclerosis (MS), HIV induced myelopathy and others).
Myelography with subsequent computed tomography of lumbar part was done. These tests showed
no herniation of the disc or neoplasm. Further examination was targeted to find the etiological basis of
the myelopathy. The urine was normal as were the
results of routine hematological, blood chemical
findings. A lumbar puncture was performed (Table).
Cerebrospinal fluid (CSF) showed pleocytosis (18
cells in mm³) with domination of lymphocytes (13
cells) and elevated protein level (0.88 g/l). A CSF
white blood cell count greater than 10 per mm3 or a
CSF protein level greater than 50 mg per dl (0.50 g/l)
indicates possible neurosyphilis.
Serologic syphilis diagnostic reactions were made,
and reagin plasma response (RPR) was positive
(1:128) showing recent infection. Treponema pallidum
hemagglutination assay (TPHA) was positive (4+).
Serum enzyme-linked immunosorbent assay (ELISA)
test was positive. Venereal Disease Research Laboratory (VDRL) test in CSF was positive, rate 1:16 (+).
HIV test was negative. Direct examination for spirochetes was not performed because perianal syphilitic
lesions were not erosive.
Table. Findings of cerebrospinal fluid on lumbar puncture
Variable
Appearance of CSF
Cells (per mm3)
Red blood cells
White blood cells
Differential count
Lymphocytes
Granulocytes
Monocytes
Glucose (mmol/l)
Total protein (g/l)
Findings
Normal range
Clear, colorless
Clear, colorless
0
18
0
<5
13
5
0
2.9
0.88
0–5 (70% of WBC)
0
0–5 (30% of WBC)
60–70% of plasma level
0.15–0.4
CSF – cerebrospinal fluid; WBC – white blood cells.
Medicina (Kaunas) 2006; 42(5)
Neurosyphilis manifesting as spinal transverse myelitis
403
A magnetic resonance imaging (MRI) examination
of the cervical and thoracic spine (Fig. 1–5) showed a
T2 hyperintense signal lesion intramedullary in the
ventral part of the spinal cord on the level of T6–7
vertebra, accumulating contrast material in T1 regime.
This is a lesion typical of myelitis.
Neuro-ophthalmological testing revealed no edema
in the retina and hyperemic veins. Argyll Robertson
symptom was not observed. Otoneurological examination and audiogram showed no abnormalities.
Adequate treatment was started. The recommendations of Centers for Disease Control and Prevention
for the treatment of neurosyphilis include 18 to 24
million IU of intravenous penicillin each day for 10
to 14 days (5). Treatment for neurosyphilis approved
by Lithuanian Ministry of Health is: benzylpenicillin
sodium 12–24 million IU/day i/v (2–4 million VV
every 4 hours for 10–21 days) or procaine benzyl-
penicillin 1.2–2.4 million IU i/m once a day for 14–
21 days. Patient received treatment with intravenous
penicillin 4 million IU five times a day, 16 days
together with prednisolone starting at 30 mg, reducing
the dose and discontinuing administration of the medication in order to prevent occurrence of Jarisch–
Herxheimer reaction. This reaction is an inflammatory
response to the destruction of treponemes (6). Symptoms of Jarisch–Herxheimer reaction are myalgia, headache, chills, tachycardia, increased respiratory rate,
and malaise. Jarisch–Herxheimer reaction occurs in
50 to 75% of people with early syphilis who receive
treatment with antibiotics (7). Despite prednisolone
administration, the patient complained of malaise,
myalgia, and headache. Patient’s temperature went up
(37.1oC) after the first injection of penicillin. Treatment showed positive effect, and patient could walk
short distances with some nursing personnel help.
Fig. 1. Sagittal slice, T1 W/TSE (TR 550 ms,
TE 13 ms) – without contrast enhancement
Fig. 3. Sagital slices, T2 W/TSE
(TR 2744 ms, TE 120 ms)
Fig. 2. Sagittal slice, T1 W/TSE (TR 550 ms,
TE 13 ms) with contrast enhancement
Fig. 4. Axial slices, T1 W/SE (TR 550 ms, TE 13
ms) duty, after contrast enhancement
Medicina (Kaunas) 2006; 42(5)
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Vaidas Matijošaitis, Antanas Vaitkus, Valius Pauza et al.
Fig. 5. Coronal slices, T1 W/SE (TR 550 ms, TE 13 ms) duty, after contrast enhancement
Urinary dysfunction remained. Condylomata lata in
anal region diminished. The patient continued treatment in another hospital where he got treatment for
another four days (4 million IU five times a day in all
20 days). RPR level diminished to 1:64 after treatment.
The patient did not show up for repeated control after
treatment.
Discussion
Neurosyphilis is a rather rare disease in our practice, especially manifesting as spinal myelitis (8). We
found only a few cases on the Internet described.
Asymptomatic syphilis occurs in 8 to 40% of infected patients (9, 10). After evaluating clinical symptoms
(papular lesions in the anus region, a scar on the penis
surface, which could possibly be after chancre, and
neurological symptoms), positive serological tests and
positive VDRL in the cerebrospinal fluid, lesions
found in the MRI of the spinal cord, diagnosis of early
secondary syphilis complicated with neurosyphilis
was made. Because epidemiological history was not
clear (patient’s girlfriend died one and a half year ago),
we could not be sure about the duration of the disease.
But specific skin lesions and our hypothesis that the
patient had sexual contact 1.5 years ago let us think
about early syphilis, because the duration of the disease is less than 2 years. Favorable effect of the treatment also supports the stage of secondary syphilis.
As we can see in this case, neurosyphilis can cause
some diagnostic, differential diagnostic and therapeutic difficulties. Spinal neurosyphilis has to be distinguished from other causes of myelitis, e.g. spinal tumor, intervertebral disc herniation, abscess, MS, HIV
induced myelopathy and others (9, 11).
The prevalence of syphilis in Lithuania decreased
over the last years (4), but there are still many things
to do to make it lower. Many centers of sexually
transmitted diseases work anonymously, and when
new cases of syphilis are diagnosed, contact persons
are not usually determined. Under such circumstances
more cases of undertreated and undiagnosed diseases
appear (12). Cases like this as well as all unclear cases
should be investigated in order to exclude neurosyphilis; serological tests should be done. All patients with
secondary syphilis or syphilis of more than one year
should be evaluated for neurosyphilis. Patients should
be monitored closely for signs and symptoms of recurrence.
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Nugaros smegenų mielitu pasireiškęs neurosifilis
Vaidas Matijošaitis, Antanas Vaitkus, Valius Pauza, Skaidra Valiukevičienė1, Rymantė Gleiznienė2
Kauno medicinos universiteto Neurologijos klinika, 1Odos ir venerinių ligų klinika,
2
Radiologijos klinika
Raktažodžiai: mielitas, neurosifilis, magnetinio rezonanso tomografija.
Santrauka. Dėl neurosifilio išsivystęs mielitas ypač reta šiuolaikinėje medicinos literatūroje mažai aprašoma
liga, labai mažai atlikta vaizdinių (branduolių magnetinio rezonanso) tyrimų. Šiame straipsnyje pateikiamas
klinikinis neurosifilio atvejis, kuris yra aktualus diagnostikos, diferencinės diagnostikos bei gydymo taktikos
požiūriu. Į gydymo įstaigą kreipėsi pacientas, besiskundžiantis progresuojančiu kojų silpnumu, tirpimu, šaudančio pobūdžio skausmu kojose bei dubens organų funkcijos sutrikimu. Neurologinio tyrimo metu nustatyti
nugaros smegenų pažeidimo simptomai: kojų raumenų silpnumas, paviršinio bei gilaus jutimo sutrikimas,
patologiniai refleksai kojose. Įtarus tarpslankstelinio disko išvaržą ar naviką, atlikta mielografija bei stuburo
kompiuterinė tomografija, tačiau minėtų patologijų nenustatyta. Po kruopštesnės paciento apžiūros (pastebėtas
randelis lytinių organų srityje, vėliau ir kondiliomos išangės srityje) įtartas sifilio sukeltas nugaros smegenų
pažeidimas. Atlikti serologiniai sifilio diagnostikos tyrimai (TPHA – angl. Treponema pallidum hemagglutination assay; RPR – angl. reagin plasma response; ELISA – angl. serum enzyme-linked immunosorbent
assay) bei smegenų skysčio tyrimai (bendras smegenų skysčio tyrimas, VDRL – angl. Venereal Disease Research
Laboratory test) patvirtino neurosifilio diagnozę. Atlikus nugaros smegenų magnetinio rezonanso tomografiją,
nustatytas nugaros smegenų pažeidimo židinys, įvertintas kaip spinalinis sifilis arba sifilinio sukeltas mielitas.
Skirtas standartizuotas gydymas buvo iš dalies veiksmingas.
Adresas susirašinėti: A. Vaitkus, KMU Neurologijos klinika, Eivenių 2, 50009 Kaunas
El. paštas: [email protected]
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